Canadian Journal of Cardiology 29 (2013) 1742.e1e1742.e3 www.onlinecjc.ca
Case Report
Very Prolonged Episode of Self-Terminating Ventricular Fibrillation in a Patient With Brugada Syndrome Stephane Boule, MD, Claude Kouakam, MD, and François Brigadeau, MD Lille University Hospital, Department of Cardiovascular Medicine, Lille, France
ABSTRACT
RESUM E
We report the case of a very prolonged spontaneous episode of self-terminating ventricular fibrillation in a patient with Brugada syndrome (BrS). The patient first underwent implantation of an internal loop recorder after an episode of prolonged loss of consciousness (several minutes) that was suggestive of a nonarrhythmic cause. After a second episode of prolonged syncope, subsequent interrogation of the loop recorder revealed a very prolonged episode of self-terminating ventricular arrhythmia, lasting 2 minutes and 41 seconds. This short report emphasizes the fact that an arrhythmic cause of syncope should not be ruled out in patients with BrS presenting with very prolonged loss of consciousness.
pisode très prolonge de fibrillation venNous rapportons le cas d’un e ment re solutive chez un patient atteint du syndrome triculaire spontane tait porteur d’un holter implantable suite de Brugada (SBr). Le patient e pisode de perte de connaissance prolonge e (plusieurs minutes) à un e tait e vocatrice d’une cause non rythmique. Après un deuxième qui e pisode de syncope prolonge e, l’interrogation des mémoires du holter e ve le un e pisode très prolonge d’arythmie ventriculaire implantable a re ment re solutive ayant dure 2 minutes et 41 secondes. Ce spontane court rapport met l’accent sur le fait qu’une cause rythmique de carte e chez les patients porteurs du SBr syncope ne doit pas être e sentant une perte de connaissance très prolonge e. pre
Case Presentation A previously healthy 33-year-old man was referred to our institution for a prolonged transient loss of consciousness (T-LOC). He had no significant medical history and was not on any medications. The T-LOC occurred at rest. According to the patient, the episode lasted several minutes and was followed by a long postsyncopal recovery period. A baseline electrocardiogram showed a slight ST-segment elevation in lead V2 with a saddle back pattern (Fig. 1A). Both echocardiogram and cardiac magnetic resonance images were unremarkable. Intravenous ajmaline challenge induced a horizontal ST-segment elevation in leads V1 and V2 placed in a normal position, with a positive T wave. A coved-type ST-segment elevation with a negative T wave was unmasked in lead V2 placed in the second intercostal space (Fig. 1B), meeting the diagnostic criteria for Brugada syndrome (BrS).1 Genetic screening for SCN5A mutations was negative. Programmed ventricular stimulation did not induce any arrhythmia. Owing to the very long duration (several minutes) of the T-LOC, which suggested a nonarrhythmic cause of the
syncope, an internal loop recorder was implanted. Eleven months later, the patient had a second syncopal episode at rest. Subsequent interrogation of the internal loop recorder revealed a very prolonged (2 minutes and 41 seconds) episode of ventricular fibrillation (VF), which secondarily organized into fast monomorphic ventricular tachycardia before spontaneously resolving into sinus rhythm (Fig. 2). Electrocardiograms recorded after this episode of VF showed a spontaneous type 1 pattern (Fig. 1C). At this point, an implantable cardioverter-defibrillator was implanted, and 6 weeks later a third syncopal episode occurred at rest, once again resulting from VF, which was terminated by a single shock. After this, treatment with oral quinidine 300 mg twice daily was started, and no further episodes of arrhythmia occurred during the following year.
Received for publication August 19, 2013. Accepted September 8, 2013. Corresponding author: Dr Stephane Boule, Pôle de Cardiologie, Hôpital Cardiologique, CHRU, 50370, Lille, France. Tel.: þ33320445038; fax: þ33320446898. E-mail: [email protected] See page 1742.e2 for disclosure information.
Discussion Risk stratification in patients with BrS presenting with syncope is difficult, and subsequent therapeutic decisions are usually challenging.2 Although episodes of self-terminating VF have been described in BrS,3,4 their duration is typically short, usually lasting no more than a few seconds. In a recent study characterizing episodes of self-terminating VF in BrS, Cismaru et al. reported an average duration of < 30 seconds3 and a maximum of 47 seconds. Therefore, until now, an arrhythmic cause of syncope has been considered very unlikely in patients with BrS when the loss of consciousness lasts > 1
0828-282X/$ - see front matter Ó 2013 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.cjca.2013.09.004
1742.e2
Canadian Journal of Cardiology Volume 29 2013
self-limiting ventricular arrhythmias in patients with BrS. This observation may have important implications regarding the initial clinical evaluation of syncope in BrS. It provides evidence that an arrhythmic cause of syncope should not be ruled out in patients with BrS presenting with very prolonged loss of consciousness. Acknowledgements The authors thank Edward Botcherby for his linguistic advice. Disclosures The authors have no conflicts to disclose. References
Figure 1. Electrocardiographic features. (A) Initial presenting electrocardiogram, showing a saddle back pattern in lead V2. (B) During ajmaline challenge, a coved-type ST-segment elevation, with a 0.3-mV J-point elevation and negative T wave, was unmasked in lead V2 positioned in the second intercostal space. (C) Electrocardiogram recorded before cardioverter-defibrillator implantation, showing a spontaneous typical type 1 pattern of Brugada syndrome. The J-point elevation (0.3 mV) is followed by a coved-type ST-segment elevation and a negative T wave in lead V2.
minute.4 However, the present case shows that very prolonged loss of consciousness (> 2 minutes) can indeed be caused by
1. Priori SG, Wilde AA, Horie M, et al. Executive summary: HRS/EHRA/ APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes. Heart Rhythm 2013. [Epub ahead of print] 2. Kubala M, Aissou L, Traulle S, Gugenheim AL, Hermida JS. Use of implantable loop recorders in patients with Brugada syndrome and suspected risk of ventricular arrhythmia. Europace 2012;14:898-902. 3. Cismaru G, Brembilla-Perrot B, Pauriah M, et al. Cycle length characteristics differentiating non-sustained from self-terminating ventricular fibrillation in Brugada syndrome. Europace 2013;15:1313-8. 4. Sacher F, Arsac F, Wilton SB, et al. Syncope in Brugada syndrome patients: prevalence, characteristics, and outcome. Heart Rhythm 2012;9: 1272-9.
et al. Boule Self-Terminating VF and Brugada Syndrome
1742.e3
Figure 2. Self-terminating episode of ventricular fibrillation (VF) recorded by the internal loop recorder. Note the prolonged duration of the arrhythmia (2 minutes and 41 seconds). The onset of the ventricular arrhythmia was preceded by a slow sinus rhythm (cycle length ¼ 1140 ms) with marked coved ST-segment elevation followed by T-wave inversion. A premature ventricular complex (with coupling interval of 380 ms) is seen triggering VF, which subsequently organizes into fast (cycle length ¼ 250 ms) monomorphic ventricular tachycardia and finally resolves spontaneously.
Case Report
Very Prolonged Episode of Self-Terminating Ventricular Fibrillation in a Patient With Brugada Syndrome Stephane Boule, MD, Claude Kouakam, MD, and François Brigadeau, MD Lille University Hospital, Department of Cardiovascular Medicine, Lille, France
ABSTRACT
RESUM E
We report the case of a very prolonged spontaneous episode of self-terminating ventricular fibrillation in a patient with Brugada syndrome (BrS). The patient first underwent implantation of an internal loop recorder after an episode of prolonged loss of consciousness (several minutes) that was suggestive of a nonarrhythmic cause. After a second episode of prolonged syncope, subsequent interrogation of the loop recorder revealed a very prolonged episode of self-terminating ventricular arrhythmia, lasting 2 minutes and 41 seconds. This short report emphasizes the fact that an arrhythmic cause of syncope should not be ruled out in patients with BrS presenting with very prolonged loss of consciousness.
pisode très prolonge de fibrillation venNous rapportons le cas d’un e ment re solutive chez un patient atteint du syndrome triculaire spontane tait porteur d’un holter implantable suite de Brugada (SBr). Le patient e pisode de perte de connaissance prolonge e (plusieurs minutes) à un e tait e vocatrice d’une cause non rythmique. Après un deuxième qui e pisode de syncope prolonge e, l’interrogation des mémoires du holter e ve le un e pisode très prolonge d’arythmie ventriculaire implantable a re ment re solutive ayant dure 2 minutes et 41 secondes. Ce spontane court rapport met l’accent sur le fait qu’une cause rythmique de carte e chez les patients porteurs du SBr syncope ne doit pas être e sentant une perte de connaissance très prolonge e. pre
Case Presentation A previously healthy 33-year-old man was referred to our institution for a prolonged transient loss of consciousness (T-LOC). He had no significant medical history and was not on any medications. The T-LOC occurred at rest. According to the patient, the episode lasted several minutes and was followed by a long postsyncopal recovery period. A baseline electrocardiogram showed a slight ST-segment elevation in lead V2 with a saddle back pattern (Fig. 1A). Both echocardiogram and cardiac magnetic resonance images were unremarkable. Intravenous ajmaline challenge induced a horizontal ST-segment elevation in leads V1 and V2 placed in a normal position, with a positive T wave. A coved-type ST-segment elevation with a negative T wave was unmasked in lead V2 placed in the second intercostal space (Fig. 1B), meeting the diagnostic criteria for Brugada syndrome (BrS).1 Genetic screening for SCN5A mutations was negative. Programmed ventricular stimulation did not induce any arrhythmia. Owing to the very long duration (several minutes) of the T-LOC, which suggested a nonarrhythmic cause of the
syncope, an internal loop recorder was implanted. Eleven months later, the patient had a second syncopal episode at rest. Subsequent interrogation of the internal loop recorder revealed a very prolonged (2 minutes and 41 seconds) episode of ventricular fibrillation (VF), which secondarily organized into fast monomorphic ventricular tachycardia before spontaneously resolving into sinus rhythm (Fig. 2). Electrocardiograms recorded after this episode of VF showed a spontaneous type 1 pattern (Fig. 1C). At this point, an implantable cardioverter-defibrillator was implanted, and 6 weeks later a third syncopal episode occurred at rest, once again resulting from VF, which was terminated by a single shock. After this, treatment with oral quinidine 300 mg twice daily was started, and no further episodes of arrhythmia occurred during the following year.
Received for publication August 19, 2013. Accepted September 8, 2013. Corresponding author: Dr Stephane Boule, Pôle de Cardiologie, Hôpital Cardiologique, CHRU, 50370, Lille, France. Tel.: þ33320445038; fax: þ33320446898. E-mail: [email protected] See page 1742.e2 for disclosure information.
Discussion Risk stratification in patients with BrS presenting with syncope is difficult, and subsequent therapeutic decisions are usually challenging.2 Although episodes of self-terminating VF have been described in BrS,3,4 their duration is typically short, usually lasting no more than a few seconds. In a recent study characterizing episodes of self-terminating VF in BrS, Cismaru et al. reported an average duration of < 30 seconds3 and a maximum of 47 seconds. Therefore, until now, an arrhythmic cause of syncope has been considered very unlikely in patients with BrS when the loss of consciousness lasts > 1
0828-282X/$ - see front matter Ó 2013 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.cjca.2013.09.004
1742.e2
Canadian Journal of Cardiology Volume 29 2013
self-limiting ventricular arrhythmias in patients with BrS. This observation may have important implications regarding the initial clinical evaluation of syncope in BrS. It provides evidence that an arrhythmic cause of syncope should not be ruled out in patients with BrS presenting with very prolonged loss of consciousness. Acknowledgements The authors thank Edward Botcherby for his linguistic advice. Disclosures The authors have no conflicts to disclose. References
Figure 1. Electrocardiographic features. (A) Initial presenting electrocardiogram, showing a saddle back pattern in lead V2. (B) During ajmaline challenge, a coved-type ST-segment elevation, with a 0.3-mV J-point elevation and negative T wave, was unmasked in lead V2 positioned in the second intercostal space. (C) Electrocardiogram recorded before cardioverter-defibrillator implantation, showing a spontaneous typical type 1 pattern of Brugada syndrome. The J-point elevation (0.3 mV) is followed by a coved-type ST-segment elevation and a negative T wave in lead V2.
minute.4 However, the present case shows that very prolonged loss of consciousness (> 2 minutes) can indeed be caused by
1. Priori SG, Wilde AA, Horie M, et al. Executive summary: HRS/EHRA/ APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes. Heart Rhythm 2013. [Epub ahead of print] 2. Kubala M, Aissou L, Traulle S, Gugenheim AL, Hermida JS. Use of implantable loop recorders in patients with Brugada syndrome and suspected risk of ventricular arrhythmia. Europace 2012;14:898-902. 3. Cismaru G, Brembilla-Perrot B, Pauriah M, et al. Cycle length characteristics differentiating non-sustained from self-terminating ventricular fibrillation in Brugada syndrome. Europace 2013;15:1313-8. 4. Sacher F, Arsac F, Wilton SB, et al. Syncope in Brugada syndrome patients: prevalence, characteristics, and outcome. Heart Rhythm 2012;9: 1272-9.
et al. Boule Self-Terminating VF and Brugada Syndrome
1742.e3
Figure 2. Self-terminating episode of ventricular fibrillation (VF) recorded by the internal loop recorder. Note the prolonged duration of the arrhythmia (2 minutes and 41 seconds). The onset of the ventricular arrhythmia was preceded by a slow sinus rhythm (cycle length ¼ 1140 ms) with marked coved ST-segment elevation followed by T-wave inversion. A premature ventricular complex (with coupling interval of 380 ms) is seen triggering VF, which subsequently organizes into fast (cycle length ¼ 250 ms) monomorphic ventricular tachycardia and finally resolves spontaneously.
